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CEN Case Rep 2021 May 4. Epub 2021 May 4.

Department of Pathology and Oncology, School of Medicine, University of Occupational and Environmental Health, 1-1 Iseigaoka, Yahatanishi-ku, Kitakyushu, Fukuoka, Japan.

Microscopic polyangiitis (MPA) is a type of necrotizing vasculitis associated with high levels of myeloperoxidase-specific antineutrophil cytoplasmic antibody (MPO-ANCA). While generally associated with renal dysfunction, MPA can also cause intraabdominal hemorrhage in rare cases. A 66-year-old man was admitted to our hospital for renal dysfunction, numbness, and weight loss for 3 months. Read More

Front Pediatr 2021 15;9:645785. Epub 2021 Apr 15.

Department of Pediatric Nephrology and Rheumatism and Immunology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, China.

The aim of this study was to explore the clinical features, pathological characteristics, and the prognosis of children with microscopic polyangiitis (MPA). Ten children with MPA that were hospitalized in our hospital were included in this study. The children's pre-diagnosis status, clinical manifestations, renal pathology, treatment, and prognosis data were analyzed retrospectively. Read More

Int J Rheumatol 2021 8;2021:5534851. Epub 2021 Apr 8.

Vasculitis Clinic, Canadian Network for Research on Vasculitides (CanVasc), Division of Internal Medicine, Hôpital du Sacré-Coeur de Montréal, University of Montreal, Montreal, Quebec, Canada.

Significant progress has been made in the treatment of ANCA-associated vasculitides (AAV), notably in granulomatosis with polyangiitis and microscopic polyangiitis. Over the past few years, many innovative studies have changed the way we now induce and maintain remission in AAV; achieving remission while limiting treatment toxicity is the key. This article provides an in-depth, up-to-date summary of recent trials and suggests treatment algorithms for induction and maintenance of remission based on the latest guidelines. Read More

J Clin Med 2021 Apr 18;10(8). Epub 2021 Apr 18.

Statistics and Epidemiology Consultant, ARJR Media LLC, Scottsdale, AZ 85259, USA.

The current data on rates and geographic distribution of vasculitis mortality are limited. We aimed to estimate the mortality rates of primary systemic vasculitis and its geographic distribution using recent population data in the United States. The mortality rates of vasculitis from 1999 to 2019 were obtained from the Center for Disease Control (CDC) Wonder Multiple Cause of Death (MCD). Read More

Diagnostics (Basel) 2021 Apr 9;11(4). Epub 2021 Apr 9.

Department of Ophthalmology, Tokyo Dental College Ichikawa General Hospital, Chiba 272-8513, Japan.

Granulomatosis with polyangiitis (GPA) presents with a variety of systemic findings, sometimes with ocular findings initially, but is often difficult to diagnose at an early stage. An 85-year-old male had complaints of ocular dryness and redness and was diagnosed with meibomian gland dysfunction with meibomitis. Despite an initial treatment with topical steroid and antibiotics, the meibomitis did not improve and the left eye developed scleritis and iridocyclitis. Read More

J Clin Med 2021 Apr 6;10(7). Epub 2021 Apr 6.

Department of Nephrology and Rheumatology, University Medical Center Göttingen, 37075 Göttingen, Germany.

Background: Renal involvement is a common and severe complication of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), potentially resulting in a pauci-immune necrotizing and crescentic ANCA glomerulonephritis (GN) with acute kidney injury (AKI), end-stage renal disease (ESRD) or death. There is recent evidence that the degree of proteinuria at diagnosis is associated with long-term renal outcome in ANCA GN. Therefore, we here aimed to systematically describe the association between proteinuria and clinicopathological characteristics in 53 renal biopsies with ANCA GN and corresponding urinary samples at admission. Read More

J Clin Med 2021 Apr 1;10(7). Epub 2021 Apr 1.

Department of Medicine, University of Cambridge, Cambridg CB2 0QQ, UK.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) represents a group of small vessel vasculitides characterized by granulomatous and neutrophilic tissue inflammation, often associated with the production of antibodies that target neutrophil antigens. The two major antigens targeted by ANCAs are leukocyte proteinase 3 (PR3) and myeloperoxidase (MPO). AAV can be classified into 3 categories based on patterns of clinical involvement: namely, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic GPA (EGPA). Read More

Curr Rheumatol Rep 2021 Apr 28;23(6):37. Epub 2021 Apr 28.

Department of Internal Medicine IV (Nephrology and Hypertension), Medical University Innsbruck, Anichstrasse 35, 6020, Innsbruck, Austria.

Purpose Of Review: There is ongoing debate concerning the classification of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. That is, whether classification should be based on the serotype (proteinase 3 (PR3)- or myeloperoxidase (MPO)-ANCA) or on the clinical phenotype (granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA)). To add clarity, this review focused on integration of the most recent literature. Read More

Curr Rheumatol Rep 2021 Apr 28;23(6):38. Epub 2021 Apr 28.

Rheumatology Department, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pz.le Golgi 2, 27100, Pavia, Italy.

Purpose Of Review: The purpose of this review is to discuss the most recent evidence on the treatment innovations and future prospective in the management of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs).

Recent Findings: In AAV, a growing body of research is available on novel treatment options for remission induction and to clarify some uncertainties concerning the optimal use of available drugs. Efforts are being made to reduce the toxicity associated with high-dose, prolonged glucocorticoids (GC) regimens. Read More

Medicina (B Aires) 2021 ;81(2):198-207

Servicio de Inmunología, Instituto de Investigaciones Médicas Alfredo Lanari, Universidad de Buenos Aires, Argentina.

ANCA-associated vasculitis is a heterogeneous group of rare autoimmune conditions of unknown cause. Clinical characteristics and prognostic factors were analyzed in 47 patients: 20 (42.5%) with granulomatosis with polyangiitis, 17 (36. Read More

Cureus 2021 Mar 23;13(3):e14069. Epub 2021 Mar 23.

Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, USA.

Microscopic polyangiitis (MPA) is an autoimmune small-vessel vasculitis often positive for perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA), or anti-myeloperoxidase (MPO), that classically affects the lungs, kidneys, and skin. Several atypical presentations of MPA involving other organs have also been reported in the literature. We report a unique case of a patient who presented with rare presentations of MPA: hearing and vision loss, dysphagia, renal dysfunction. Read More

Arthritis Rheumatol 2021 Apr 21. Epub 2021 Apr 21.

Department of Rheumatology, National Reference Center for Systemic Autoimmune Diseases (CNR RESO), Hôpitaux Universitaires de Strasbourg, F-67000, Strasbourg, France.

Objective: Silica is one of the strongest environmental substances linked with autoimmunity. The aim of this study was to assess the prevalence of granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and renal limited vasculitis (RLV) in a French northeast region, and their geospatial association with quarries.

Methods: Potential ANCA-Associated Vasculitis (AAV) cases were identified using three sources: hospital records, immunology laboratories and the National Health Insurance System. Read More

RMD Open 2021 Apr;7(2)

Glasgow Renal and Transplant Unit, Queen Elizabeth University Hospital, Glasgow, UK.

Background: Small studies suggest an association between ANCA-associated vasculitis (AAV) incidence and rurality, seasonality and socioeconomic deprivation. We examined the incidence of kidney biopsy-proven AAV and its relationship with these factors in the adult Scottish population.

Methods: Using the Scottish Renal Biopsy Registry, all adult native kidney biopsies performed between 2014 and 2018 with a diagnosis of granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) were identified. Read More

Autoimmun Rev 2021 Apr 16;20(6):102829. Epub 2021 Apr 16.

Department of Biomedicine, Aarhus University, Aarhus, Denmark; Department of Rheumatology, Aarhus University Hospital, Aarhus, Denmark; Department of Rheumatology, Silkeborg Regional Hospital, Silkeborg, Denmark. Electronic address:

The primary vasculitides constitute a heterogeneous group of immune mediated diseases of incompletely understood pathogenesis currently classified by the size of blood vessels affected (Chapel Hill classification). In recent years, several drugs with well-characterized immunological targets have been tested in clinical trials in large vessel vasculitis and small vessel vasculitis. Such trials provide "reverse translational" or bedside to bench information about underlying pathogenic mechanisms. Read More

Front Immunol 2021 31;12:629902. Epub 2021 Mar 31.

Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea.

Objective: Evidences indicate that the risk of stroke is increased in autoimmune rheumatic diseases. This study aimed to investigate the incidence of stroke in patients with systemic necrotizing vasculitis (SNV) using the national health database.

Methods: Data were obtained from the Korean National Claims database between 2010 and 2018 to identify incident SNV [anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) and polyarteritis nodosa (PAN)] cases. Read More

Medicine (Baltimore) 2021 Apr;100(15):e24889

Department of Rheumatology.

Rationale: Anti-myelin oligodendrocyte protein antibody-associated disease (MOGAD) is a new disease entity with various clinical phenotypes. MOGAD often present with recurrent optic neuritis (ON), and it can also develop as a compartment of neuromyelitis optica spectrum disorder (NMOSD). Moreover, multiple autoantibodies such as an anti-myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) had been reported in the serum of patients with NMOSD. Read More

BMJ Case Rep 2021 Apr 12;14(4). Epub 2021 Apr 12.

Division of Rheumatology, Department of Medicine, UF Health Jacksonville, Jacksonville, Florida, USA.

We describe a case of a 48-year-old woman who presented with acute respiratory failure due to diffuse alveolar haemorrhage and acute renal failure due to pauci-immune glomerulonephritis consistent with a new diagnosis of microscopic polyangiitis (MPA). The patient had a recent SARS-CoV-2 infection 6 weeks before MPA diagnosis and had stopped immunosuppression for her rheumatoid arthritis (RA) at that time. The patient was treated with pulse intravenous steroids, plasma exchange therapy and rituximab, which induced remission of her illness. Read More

J Clin Rheumatol 2021 Mar 25. Epub 2021 Mar 25.

From the Division of Rheumatology, Allergy, and Clinical Immunology Division of Infectious Diseases and Global Medicine Department of Pathology, Immunology, and Laboratory Medicine Department of Dermatology Division of Pulmonary and Critical Care Medicine, University of Florida, Gainesville, FL.

J Clin Rheumatol 2021 Apr 9. Epub 2021 Apr 9.

From the Department of Rheumatology, Hospital Nacional Guillermo Almenara Irigoyen, EsSalud, Lima Universidad Científica del Sur, Villa El Salvador Unidad de Investigación para la Generación y Síntesis de Evidencias en Salud, Universidad San Ignacio de Loyola Universidad Nacional Mayor de San Marcos, Lima, Peru Division of Clinical Immunology and Rheumatology, Department of Medicine, School of Medicine, The University of Alabama at Birmingham, Birmingham, AL Department of Medicine, School of Medicine, Universidad Peruana Cayetano Heredia, Lima, Peru.

Aim: To validate the new classification criteria for antineutrophil cytoplasmic antibody-associated vasculitis in a real-life Peruvian cohort of antineutrophil cytoplasmic antibody-associated vasculitis patients.

Methods: We reviewed medical records from a Peruvian tertiary care center from January 1990 to December 2019. Antineutrophil cytoplasmic antibody-associated vasculitis was diagnosed based on the 1990 American College of Rheumatology (ACR) criteria, the 2012 Chapel Hill Consensus Conference definitions, the European Medicines Agency (EMEA) algorithm, and the clinical acumen of the treating rheumatologists. Read More

Front Immunol 2021 26;12:624848. Epub 2021 Mar 26.

Institute of Dermatology and Department of Dermatology, The First Affiliated Hospital, Anhui Medical University, Hefei, China.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is characterized by the inflammation of small and medium vessels and presence of proteinase 3-ANCA or myeloperoxidase-ANCA in the circulation. AAV comprises three clinical subtypes: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic GPA (EGPA). Although the pathogenesis of AAV is still unclear, genetic and environmental factors and the immune system are thought to be involved. Read More

J Neurol Sci 2021 May 27;424:117423. Epub 2021 Mar 27.

Burden Professor of Clinical Neurosciences, University of Bristol Institute of Clinical Neurosciences, UK, Gulu University Faculty of Medicine, Uganda. Electronic address:

Both the CNS and the PNS can be involved in almost all of the vasculitides - including the primary systemic vasculitic disorders, such as microscopic polyangiitis and polyarteritis nodosa, and in non-vasculitic systemic disorders, such as systemic lupus erythematosis and sarcoidosis. The latter diseases also include infections and toxininduced disorders - particularly drugs of abuse such as cocaine and amphetamines. Here we will summarise the spectrum of these disorders as they affect the CNS, concentrating in particular on their distinguishing clinical and investigational features. Read More

BMC Immunol 2021 Apr 12;22(1):26. Epub 2021 Apr 12.

Department of Rheumatology and Clinical Immunology, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg im Breisgau, Germany.

Background: Cytotoxic Natural Killer (NK) cells are increasingly recognized as a powerful tool to induce targeted cell death in cancer and autoimmune diseases. Still, basic blood NK cell parameters are poorly defined. The aims of this study were 1) to establish reference values of NK cell counts and percentages in healthy adults; 2) to describe these parameters in the prototype autoimmune disease group ANCA-associated vasculitis (AAV); and 3) to investigate whether NK cell counts and percentages may be used as activity biomarkers in the care of AAV patients, as suggested by a preceding study. Read More

Rom J Ophthalmol 2021 Jan-Mar;65(1):93-97

Emergency Eye Hospital, Bucharest, Romania.

To describe the chronology and the extent of orbital involvement in a case of granulomatosis with polyangiitis. Descriptive case report and literature review. A 45-year-old patient, formerly diagnosed with granulomatosis with polyangiitis due to otorhinolaryngologic manifestations, pulmonary lesions, renal impairment, left knee arthritis and high blood levels of antineutrophil cytoplasmic antibodies, addressed the Ophthalmology Department in November 2020, having the following complaints: left eye mild retro-orbital discomfort, proptosis and epiphora. Read More

Rom J Ophthalmol 2021 Jan-Mar;65(1):10-14

Cluj County Emergency Hospital, Cluj-Napoca, Romania.

ANCA+ associated vasculitis (AAV) is a group of rare diseases with potentially vision-threatening complications. Ocular and orbital complications of these diseases are caused by vasculitis of the small vessels of the eye or by granulomatous mass formation. ANCA (anti-neutrophil cytoplasmic antibodies) represent a key component of pathophysiological pathways as well as a diagnostic marker. Read More

J Clin Med 2021 Mar 16;10(6). Epub 2021 Mar 16.

Department of Nephrology and Rheumatology, University Medical Center Göttingen, 37075 Göttingen, Germany.

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic vasculitis, most frequently presenting as microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA). Kidney involvement is a common and severe complication of ANCA AAV which is observed in a considerable subset of patients, mainly affecting glomeruli. However, tubulointerstitial lesions have also been described in ANCA glomerulonephritis (GN). Read More

BMJ Case Rep 2021 Mar 31;14(3). Epub 2021 Mar 31.

Internal Medicine, Rashid Hospital, Dubai Health Authority, Dubai, UAE.

A 54-year-old Indian female presented to the hospital with a 4-day history of fever, shortness of breath and blood-streaked sputum. Chest radiograph showed consolidation and she was admitted as a case of bilateral bronchopneumonia. She was started on broad-spectrum antibiotics and antivirals but she continued to deteriorate clinically with increasing oxygen requirement and worsening lung infiltrates. Read More

Rheumatology (Oxford) 2021 Mar 31. Epub 2021 Mar 31.

Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada.

Objectives: This study describes the spectrum and initial impact of pulmonary manifestations in the primary systemic vasculitides.

Methods: Description and comparison of pulmonary manifestations in adults with Takayasu's arteritis (TAK), giant cell arteritis (GCA), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), polyarteritis nodosa (PAN), and IgA vasculitis (IgAV), using data collected within the Diagnostic and Classification Criteria in Vasculitis (DCVAS) study.

Results: Data from 1952 patients with primary vasculitides were included: 170 TAK, 657 GCA, 555 GPA, 223 MPA, 146 EGPA, 153 IgAV, and 48 PAN. Read More

Rheumatol Int 2021 Mar 29. Epub 2021 Mar 29.

Oregon Health and Science University, 3181 SW Sam Jackson Park Road, Portland, OR, 97239, USA.

Diffuse alveolar hemorrhage (DAH) is a rare but potentially life-threatening emergency that has both immune and non-immune etiologies. The objective of this investigation was to compare the risk factors and outcomes of immune and non-immune causes of DAH at a tertiary-care academic center. This was a retrospective observational study conducted at a University center. Read More

World J Methodol 2021 Mar 20;11(2):15-22. Epub 2021 Mar 20.

Department of Ear, Nose and Throat, Royal National ENT and Eastman Dental Hospitals, London WC1E 6DG, United Kingdom.

Olfactory dysfunction (OD) has been described in patients with antineutrophil cytoplasmic antibody-associated vasculitides (AAV), but the underlying mechanisms are not completely understood. The causes of altered smell function can generally be divided into conductive, sensorineural or others. To date no specific treatment is available for AAV-related OD and the efficacy of currently available options has not been explored. Read More

Pediatr Transplant 2021 Mar 27:e14013. Epub 2021 Mar 27.

Division of Nephrology, Department of Pediatrics, LSU Health New Orleans, New Orleans, LA, USA.

The negative impact of COVID-19 on adults with underlying chronic kidney disease, including kidney transplant recipients, has been well documented. Children have a less severe presentation and better prognosis compared to adults. However, little is known regarding the spectrum of COVID-19 infection in children and adolescents with underlying autoimmune disorders necessitating solid organ transplant and long-term immunosuppressive therapy. Read More